ID   2.4.1.225
DE   N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase.
AN   heparan glucuronyltransferase II.
AN   N-acetylglucosaminylproteoglycan beta-1,4-glucuronyltransferase.
CA   3-O-{alpha-D-GlcNAc-[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n)-(1->4)-
CA   beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-
CA   seryl-[protein] + UDP-alpha-D-glucuronate = 3-O-{[(1->4)-beta-D-GlcA-
CA   (1->4)-alpha-D-GlcNAc](n+1)-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-
CA   beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein] + H(+) + UDP.
CC   -!- Involved in the biosynthesis of heparin and heparan sulfate.
CC   -!- Some forms of the human enzyme (particularly the enzyme complex
CC       encoded by the EXT1 and EXT2 genes) act as bifunctional
CC       glycosyltransferases, which also have the EC 2.4.1.244 activity
CC       required for the synthesis of the heparan sulfate disaccharide
CC       repeats.
DR   Q5IGR8, EXT1A_DANRE;  Q5IGR7, EXT1B_DANRE;  Q5IGR6, EXT1C_DANRE;
DR   A5D7I4, EXT1_BOVIN ;  Q9JK82, EXT1_CRIGR ;  Q9V730, EXT1_DROME ;
DR   Q16394, EXT1_HUMAN ;  P97464, EXT1_MOUSE ;  A9X1C8, EXT1_PAPAN ;
DR   Q5RBC3, EXT1_PONAB ;  O01705, EXT2_CAEEL ;  Q9Y169, EXT2_DROME ;
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